Ebstein's anomaly and left proximal pulmonary artery stenosis complicated by pulmonary embolism: a case report

Snezana Bongard, Marina Jurukovska, Irina Tonovska, Dushko Mihajlovski, Salis Tager
2013 Cardiologia Croatica  
2013;8(9):302. Cardiologia CROATICA The clinical course of Ebstein's anomaly patients varies considerably. Commonly associated cardiac structural defects include: ASD, PFO, pulmonary stenosis and VSD. We present a case of a 26-year-old patient admitted with dyspnea, exercise intolerance, epistaxis and tachycardia deteriorating for 4 months prior to admission. In a CT angiography performed prior to admission, a stenosis of the proximal left pulmonary artery was noted with a post-stenotic
more » ... on and a well developed bronchial circulation. A trans-thoracic echo upon admission showed a typical image of Ebstein's anomaly with the septal leaflet of the tricuspid valve displaced into the RV cavity, attached to a hypertrophied myocardium. Severe eccentric tricuspid regurgitation was present. Right heart catheterization measured a low pulmonary pressure of 30 mmHg. The catheter was blocked at the proximal left pulmonary artery. A CT angiography revealed a complete occlusion of the left pulmonary artery. In surgery, the septal leaflet of the tricuspid valve was dysplastic and non-functional with an abnormally high insertion of both the septal and posterior leaflets. The patient underwent pulmonary thrombo-endarterectomy, RVOT myotomy and tricuspid valve replacement with a mechanical valve ATS 31 mm. Histologically, the excised leaflets contained fibrinoid necrosis. The postoperative course was uneventful and the patient was discharged on the 7 th postoperative day and is doing well since (two months). We conclude that the patient underwent an acute event of pulmonary embolism with a background congenital pulmonary artery stenosis and Ebstein's anomaly. This case is significant for the unusual association of two rare congenital entities not known to be related. KEYWORDS: Ebstein's anomaly, pulmonary artery stenosis, pulmonaly embolism. CITATION: Cardiol Croat. 2013;8(9):302. VALVULAR HEART DISEASE Proπireni saaeetak / Extended abstract Ebstein's anomaly and left proximal pulmonary artery stenosis complicated by pulmonary embolism: a case report
doi:10.15836/ccar.2013.302 fatcat:rmtrcegdsfbwtjczqjzjwmrvpm